Sir, superior rectus sr to levator palpebrae superioris lps synkinesis has recently been described in patients with congenital or longstanding ptosis. The degree of ptosis was documented by measuring the height of each palpebral fissure in primary gaze while restraining. Versaci md, in plastic surgery secrets plus second edition, 2010. It is important to note that ptosis can be a symptom of other issues, so it would be wise to rule these out before you pursue correction. Although a vast majority of the ptosis is senile in origin, a certain percentage of the cases are associated with. Upper eyelid ptosis revisited abstract blepharoptosis, commonly referred to as ptosis is an abnormal drooping of the upper eyelid. Clinical and etiological aspects of ptosis at university. Congenital ptosis abnormal drooping of the upper eyelid, present at birth or within the first year of life is termed congenital ptosis. Ptosis is considered congenital if present at birth or if it is diagnosed within the first year of life. In some cases, ptosis can restrict and even block normal vision. In this study, we describe a case of moderate congenital palpebral ptosis in a 9yearold patient who presented with a good eyelid levator muscle function in the left eye.
The most common and resolutive approach to the treatment of congenital ptosis and its phenotypical presentation is surgical intervention. Congenital ptosis is a rare condition characterized by lower positioning of the upper eyelid that is present at birth and is a clinical condition that is persistent if not treated. Oct 30, 20 normal 910mm in primary gaze should be seen in up gaze, down gaze and primary gaze amount of ptosis difference in palpebral apertures in unilateral ptosis or difference from normal in bilateral ptosis 49. Background the frontalis suspension technique is the surgical method of choice in patients with ptosis and a levator function of 4 mm or less. The association of congenital ptosis and congenital heart disease david c. Congenital ptosis affects a child from birth and is commonly due to a defect in the levator muscle which raises the eyelids. You can see an ophthalmologist for a complete evaluation. When the eyelid elevators do not function or if there is little drive to lift the. Ptosis may be the presenting sign or symptom of serious neurologic disease.
Aesthetic surgery journal aesthetic plastic surgery, inc. Ptosis results from a congenital or acquired weakness of the levator palpebrae superioris. May 21, 2019 mild cases of congenital ptosis can be observed if there are no amblyopia, strabismus, or abnormal head posture. They identified 107 cases of ptosis, with an incidence of 7. Congenital ptotic eyelid remains higher in downgaze due to lid lag.
Congenital ptosis is the failure of neuronal migration evaluation and management of unilateral ptosis and avoiding contralateral ptosis christopher i. If eyelid closure is complete in infancy, occlusion amblyopia will occur, and this may require urgent attention. Patients with congenital ptosis may have a poorly developed lid crease. Dylan dn chan acquired ptosis is a commonly seen condition. We report a combined sr and lr to lps synkinesis in a patient with congenital ptosis. More than 100 techniques for the treatment of ptosis have been reported46. This muscle is initially formed from the superior rectus muscle during embryogenesis and reaches its normal position around the fourth month of pregnancy. Ptosis ptosis is the term used for drooping of the upper eyelids. Congenital congenital oculomotor nerve palsy aplasia. Distribution related to the degree of ptosis the upper palpebral fold was absent in 38. If your diagnosis is primary ptosis, then surgical correction is an easy process that can be done awake or under sedation. About europe pmc funders joining europe pmc governance roadmap outreach.
Congenital ptosis includes the isolated typethe congenital cranial. Congenital ptosis is more common than acquired ptosis. Current techniques in surgical correction of congenital ptosis. Oct 08, 2016 we examined the effect of surgical repair on the pattern of refractive errors in korean patients with congenital blepharoptosis. If congenital ptosis obscures any part of the pediatric patients visual field, surgery must be performed to correct the problem early in life. Blepharoptosis, or ptosis of the eyelid, refers to drooping of the upper eyelid that usually results from a congenital or acquired abnormality of the muscles that elevate the eyelid. Ptosis was defined in bilateral cases as marginal reflex distance of 1 mm lower on one side. Knowledge of the anatomy and the normal physiology of the eyelid makes it easier to understand the various ways in which ptosis may present. The autism dysmorphology measure adm was developed to provide an efficient and reliable method to identify children with general dysmorphology on physical examination. Pdf on dec 1, 2011, kate ahmad and others published ptosis find, read and cite all the. Improved visual acuity after frontalis sling surgery for. Correction of congenital ptosis is one of the most difficult challenges ophthalmologists face.
Berke 88% smith 67% and beard 62%1 simple congenital ptosis is due primarily to. Congenital ptosis may occur through autosomal dominant inheritance. In the setting of severe unilateral ptosis, the ptotic lid position can be very amblyogenic, and early surgery is advised. In almost 75 percent of the cases only one eye is affected. Pdf blepharoptosis ptosis is a common but often overlooked sign that may serve as a. The underlying cause, degree of ptosis, amount of levator function, position of the globe, presence of a bells phenomenon, and position and contour of the upper lid. The drooping may be worse after being awake longer when the individuals muscles are tired. In adults, ptosis is usually acquired due to advanced age, trauma, muscle diseases or neurological disorders. Nov 12, 2017 simple congenital ptosis blepharophimosis. Although this can be purely a cosmetic problem, it can also prevent normal visual development, so surgery to correct the lid position is sometimes necessary. In congenital ptosis, the ptotic lid appears higher in downgaze. Trauma at birth such as from the use of forceps eye movement disorders.
Palpebral ptosis indicates the abnormal drooping of the upper lid, caused by partial or total reduction in levator muscle function. In a larger study of 155 congenital ptosis, berrybrincat et al performed 15 fasanellaservat procedures. In most cases of congenital ptosis, the problem is isolated and does not affect the vision. Ptosis, also referred to as blepharoptosis, is defined as an abnormal lowlying upper eyelid margin with the eye in primary gaze. Cfeom can be categorized into eight complex strabismus of cfeom1a, cfeom1b, cfeom2, cfeom3a, cfeom3b, cfeom3c, cfeom4 tukel syndrome, and cfeom5 54,55,56,57,58,59,60,61. Congenital ptosis may be associated with superior rectus dysfunction or marcus gunn jaw winking phenomena. The patient underwent surgical treatment with the lester jones technique, as indicated for cases of moderate ptosis, and achieved satisfactory aesthetic and functional results. The frequency of congenital ptosis in the united states has not been reported. Ptosis refers to vertical narrowing of the palpebral fissure secondary to drooping of the upper eyelid to a lower than normal position. Congenital fibrosis of the extraocular muscles cfeom is characterized by congenital restrictive ophthalmoplegia, more severe in vertical eye movement, with or without ptosis. If a droopy eyelid is present at birth or within the first year of life, the condition is called congenital ptosis.
If severe enough and left untreated, the drooping eyelid can cause other conditions, such as amblyopia or astigmatism. Recently, a goretex sheet wider than a sling or strip suspension was reported. Blepharoptosis or ptosis, as it is more commonly known, is a common clinical sign that may affect individuals of all ages ranging from neonates to elderly individuals. Blepharoptosis congenital and traumatictypes of operation indicated for cogenital and acquired ptosis. Congenital ptosis associated with combined superior rectus.
Lps muscle elevates the eyelid, and biopsies of this. The overall success rate was 71%, with a 20% reintervention rate. Controversies and advances in the management of congenital ptosis. Regardless of the etiology, when ptosis obstructs vision, it is disabling. In case of mild ptosis, the eye has an undesirable appearance but is functionally normal. Congenital ptosis is the failure of neuronal migration within the levator muscle complex. Congenital ptosis in this condition, an infant is born with ptosis because of a developmental problem involving the muscle that raises the upper. It is easy to overcorrect an acquired ptosis and to undercorrect a congenital ptosis. May 21, 2019 the vertical palpebral fissure height with each eye fixating in the distance can also be recorded. Ptosis and a motility disturbance may be observed in oculomotor paresis, myasthenia gravis, chronic progressive external ophthalmoplegia, or duanes syndrome. Congenital ptosis drooping eyelid clinical presentation. Common familial occurrences suggest that genetic or chromosomal defects are likely.
Ptosis may be present at birth, or may be acquired later in life. A nerve problem in the eyelid can also cause it to droop. The lid may droop only slightly, or it may cover the pupil or eye entirely. Patients who are born with this disfigurement often have malformed muscle tissues in the eyelid or fatty deposits that lack elasticity. Number % neurogene 6 10,7 myogene 12 21,4 aponevrotic 14 25 traumatic 24 42,9 total 56 100 table 5. Ancillary testing corneal sensitivity should be tested in all cases. The rightsided image shows a normal eyelid crease a, upper marginal refl ex. Palpebral fissure an overview sciencedirect topics. Constant ptosis pseudo ptosis globe anomaly enophthalmos lid anomaly blepharochalasis dermatochalasis eye movement disorder hypertropia downgaze palsy supranuclear cortical neuroophthalmology. Blepharoptosis, the fallen eyelid, is a clinical sign that neurologists encounter regularly. This condition consists of short palpebral fissures, congenital ptosis, epicanthus inversus, and telecanthus. Lisman, md abstract treating unilateral ptosis can be challenging and a proper preoperative evaluation may help prevent unexpected outcomes on the contralateral lid. Depending on the severity of the congenital ptosis, patients should be monitored every 312 months for signs of amblyopia due to congenital ptosis. Rbcp treatment of congenital ptosis by using the lester.
Evaluation and management of unilateral ptosis and. Congenital ptosis often results from a failure in the embryonic development of the lps muscle. Childhood ptosis is not usually associated with family history or any other disease. The mother stated that the eyelid had been droopy since birth and that it appeared to be getting. When the pain is more severe, the eye is kept tightly closed blepharospasm, especially when the pain originates from the cornea, the iris, or the ciliary muscle. Pediatric congenital ptosis, while commonly encountered by the pediatric. In cases with no other reason for amblyopia, stimulus deprivation amblyopia due to ptosis was assumed. Ptosis assessment measurements were palpebral aperture pa7 mm, levator function lf twophase procedure as suggested by jones and.
Plastic and reconstructive surgery of the eye and adnexa. The association of congenital ptosis and congenital heart. Clinical presentation and management of congenital ptosis. Discussion the etiopathogenesis of congenital palpebral ptosis is the deficiency of striated muscle fibers of the levator muscle that occurs during the embryonic stage, the degree of which will determine ptosis severity. Blepharoptosis, or ptosis of the eyelid, refers to drooping of the upper ey elid that usually results from a congenital or acquired abnormality of the muscles that elevate the eyelid. Clinical presentation and management of congenital ptosis europe. Congenital ptosis, or dysmyogenic ptosis, is the most common ptosis seen in childhood. Cohen 1972 described a large family with hereditary congenital ptosis. The amount of ptosis was mild in 5 6%, moderate in 26 31%, and severe in 52 63% of 83 eyes with congenital ptosis. However, in approximately 70% of known cases, congenital ptosis affects only one eye. This condition is sometimes called lazy eye, but that term normally refers to the condition amblyopia.
This mainly happens if an inaccurate diagnosis and assessment is made or an unsuitable procedure is tried. Congenital ptosis is a rare condition characterized by lower. It may be caused by various pathologies, both congenital and acquired. Congenital ptosis treatment of ptosis when to treat. The grade of ptosis, the eyelid function, and the amblyopic risk are the parameters that. A large pupil on the side of the ptosis may indicate a cn iii palsy or post. This patient presented bilateral palpebral ptosis and dysphagia and underwent. Outcome of levator resection in congenital ptosis with poor. This gives the impression of a short palpebral fissure. The presence of significant dysmorphology is an indicator that embryogenesis did not proceed normally. Crawford technique was effective in most cases and with similar results using both materials. Today, the radiologist, to do justice to his patient and to the branch of medicine which he serves, must be well acquainted with current medical knowledge and must keep in touch not only with his colleagues, but must have a liaison with other branches of medicine and surgery. Simple congenital ptosis predominated, either unilateral or severe. The underlying cause, degree of ptosis, amount of levator function, position of the globe, presence of a bells phenomenon, and position and contour.
It comprises a group of diseases in which the ptosis is due to a developmental dystrophy of the levator. Although this condition may be the result of trauma at. As a result of that failure, fibrous and adipose tissues are present in the muscle belly rather than normal muscle fibers, diminishing the ability of the levator to contract and relax. Ptosis in infants and children is often due to a problem with the muscle that raises the eyelid. All patients included were diagnosed as congenital ptosis. Simple congenital ptosis is the most frequent type of ptosis in children. Otherwise, surgery for ptosis is often performed in later childhood. In ptosis surgery, a good cosmetic outcome is very important, this holds true for congenital myogenic ptosis as well. According to the dictionary, ptosis means the prolapse of an organ or part. Refractive error characteristics in patients with congenital. Jun 15, 2006 congenital ptosis, or dysmyogenic ptosis, is the most common ptosis seen in childhood.
If severe and not corrected in a timely manner, congenital ptosis can lead to amblyopia and permanent vision loss. Ptosis may involve one or both the eyes and ranges from mild to severe. Congenital shortening of the palpebral fissure is encountered occasionally, often in association with entropion of the upper. The most used classification, aimed at surgical planning, considers the degree of ptosis as mild, moderate, and severe when the upper eyelid margin is 24 mm below the. Congenital ptosis and congenital heart disease are known to occur simultaneously in the presence of several wellrecognized clinical syndromes. A 7 monthold male patient was referred to the oculoplastics clinic at the university of iowa by his pediatric ophthalmologist for evaluation of left upper eyelid ptosis. It is used primarily for congenital ptosis, but also for blepharophimosis syndrome and neurogenic ptosis third cranial nerve palsy and marcus gunn syndrome. Ptosis of the upper lid can be classified as congenital, neurogenic, myogenic, aponeurotic, or mechanical. Correction of congenital ptosis is one of the most difficult. Other potential causes of congenital ptosis include. In congenital ptosis the muscle that elevates the lid, called the levator palpebrae superioris, is usually absent or imperfectly developed. Congenital ptosis can vary in severity, and while mild cases may have minimal implications to visual function, more severe ptosis can lead to astigmatism causing refractive amblyopia, deprivation. Normal 910mm in primary gaze should be seen in up gaze, down gaze and primary gaze amount of ptosis difference in palpebral apertures in unilateral ptosis or difference from normal in bilateral ptosis 49. Conclusions correction of congenital ptosis using the frontalis.
Normally, upper lid covers about upper onesixth of the cornea i. Asymmetry in brow height was defined as a difference of 1 mm. Original article outcome of levator resection in congenital. It is of two types, namely congenital ptosis and acquired ptosis.
On the other hand, dukeelder 1963 stated that at least 8 types of congenital ptosis are recognizable, of. Symptoms are usually observed before the childs first birthday. If the palpebral ptosis is congenital in nature, it may or may not be noticeable at birth. Upper eyelid ptosis revisited the position of the ptotic eyelid in downgaze should be assessed, as it helps in differentiating congenital from acquired ptosis. Congenital ptosis associated with combined superior rectus, lateral rectus, and levator palpebrae synkinesis. Regardless of the etiology, when ptosis obstructs vision, it. Ptosis is considered congenital if present at birth or if it. The primary indications for treatment of any form of congenital ptosis are amblyopia and abnormal head positioning.
Eyelid ptosis can be classified as congenital or acquired, and this. In children, however, ptosis is usually congenital present at birth and related to poor eyelid muscle development. This condition can be congenital or acquired, and it may be associated with amblyopia or astigmatism. At this position there is an increased pseudo ptosis and narrowing of the palpebral aperture blepharoptosis can be classified based on its etiology into the following. In this family, which provided a sample of 37 meioses, engle et al. Drooping of the left upper lid history of present illness. Ptosis refers to a drooping or inferior displacement of the upper eyelid with associated narrowing of the vertical palpebral fissure. In addition, widening of the palpebral fissures on the contralateral side can give the appearance of pseudoptosis and may be due to eyelid retraction from graves disease, axial proptosis, congenital eyelid retraction, or high myopia. A gene for isolated congenital ptosis maps to a 3cm. Pdf frontal suspension for congenital ptosis using an. The aetiology of ptosis can be divided into structural abnormalities affecting the eyelid muscles andor surrounding tissues in the orbit, myogenic causes. This condition has multiple etiologies and is seen in all age groups.
807 232 762 545 265 1296 474 1093 1529 678 1469 1119 917 1522 278 1202 381 559 889 849 722 491 1211 210 1372 998 1038 935 952 130 1449 273 1101 114